Current Concepts in the Treatment of Early Onset Scoliosis.

Despite many surgical advances in the treatment of early onset scoliosis (EOS) over the past two decades, this condition remains a challenge to address. While otherwise healthy children can have EOS, many of these patients have complicated comorbidities making proper treatment algorithms extraordinarily difficult. Non-operative measures can be successful when initiated early, but are many times utilized as a delay tactic until growth-friendly operative procedures can be safely performed.

Reanalysis of RNA sequencing data ends diagnostic odyssey and expands the phenotypic spectrum of congenital titinopathy.

Although next-generation sequencing has enabled diagnoses for many patients with Mendelian disorders, the majority remain undiagnosed. Here, we present a sibling pair who were clinically diagnosed with Escobar syndrome, however targeted gene testing was negative. Exome sequencing (ES), and later genome sequencing (GS), revealed compound heterozygous TTN variants in both siblings, a maternally inherited frameshift variant [(NM_133378.

Outcomes and Complications in Management of Congenital Myopathy Early-Onset Scoliosis.

Background: Congenital myopathies (CMs) are complex conditions often associated with early-onset scoliosis (EOS). The purpose of this study was to investigate radiographic outcomes in CM patients undergoing EOS instrumentation as well as complications. Secondarily, we sought to compare these patients to a population with higher prevalence, cerebral palsy (CP) EOS patients.

Growth Modulation by Stimulating the Growth Plate: A Pilot Study.

This study investigates the ability of low-intensity pulsed ultrasound (LIPUS) or direct injection of recombinant growth hormone (rGH) to stimulate local growth of long bones. In a randomized controlled animal trial, healthy immature rabbits were allocated to 1 of the following 4 conditions: epiphyseal rGH periosteal injection, transdermal LIPUS, saline periosteal injection, or no treatment. New bone deposition was labeled with calcein at days 1 and 18, and microscopic measurements of growth were conducted by blinded observers.

Image quality of EOS low-dose radiography in comparison with conventional radiography for assessment of ventriculoperitoneal shunt integrity.

Objective: Patients with shunted hydrocephalus often accumulate high levels of radiation over their lifetimes during evaluation of hardware integrity. Current practice involves the use of a series of conventional radiographs for this purpose. Newer low-dose EOS radiography is currently used to evaluate scoliosis but has not been explored to evaluate shunt integrity on a large scale.

Impact of growth friendly interventions on spine and pulmonary outcomes of patients with spinal muscular atrophy.

Purpose: Patients with spinal muscular atrophy (SMA) are often treated with growth friendly devices such as vertical expandable prosthetic titanium rib(VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, thorax morphology and pulmonary outcomes.

Methods: This is a retrospective analysis of prospectively collected data.

Implant Distribution Versus Implant Density in Lenke Type 1 Adolescent Idiopathic Scoliosis: Does the Position of the Screw Matter?

Study Design: Retrospective study.

Objective: Previous studies have demonstrated that increased implant density (ID) results in improved coronal deformity correction. However, low-density constructs with strategically placed fixation points may achieve similar coronal correction.

Effect of Instrument Navigation on C-arm Radiation and Time during Spinal Procedures: A Clinical Evaluation.

Introduction: As minimally invasive spine surgery gains popularity, a focused effort must be made to reduce intraoperative radiation exposure to levels as low as reasonably achievable. Here, we demonstrate the clinical efficacy of a novel technology to aid in instrument navigation that aims to reduce intraoperative radiation exposure, number of fluoroscopic images, and time required to perform the most radiation intensive portions of a multitude of spinal procedures.

Methods: An internally randomized controlled study was performed over a 1-month period in order to clinically evaluate the effect of the C-arm assisted instrument tracking system, TrackX, on surgeon workflow, time, and radiation emitted.

Staying ahead of the curve: the use of spinopelvic parameters to predict curve progression and bracing success in adolescent idiopathic scoliosis.

Study Design: Retrospective cohort study.

Objectives: To investigate radiographic sagittal and spinopelvic parameters of patients with adolescent idiopathic scoliosis (AIS) treated with bracing and assess differences among those treated successfully and unsuccessfully. AIS is a three-dimensional deformity of the spine, sharing an intricate relationship with pelvic morphology.

Complications After Pediatric Hip Fractures: Evaluation and Management.

Pediatric proximal femur fractures are high-energy injuries with predictable and morbid complications. Osteonecrosis of the femoral head is the most common complication with identified risk factors including fracture type, patient's age, degree of displacement, timing to reduction, and stability of fixation. Additional complications include nonunion, coxa vara, and premature physeal arrest.

Periapical Wires Result in Less Curve Correction Than Pedicle Screw Constructs in Idiopathic Scoliosis.

Study Design: Retrospective study.

Purpose: The objective of this study was to compare percent correction between apical and periapical pedicle screw (PS) and sublaminar wire constructs for Cobb correction and coronal balance correction.

Overview Of Literature: The current gold standard for deformity correction in adolescent idiopathic scoliosis (AIS) are PS constructs.

Gradual Deformity Correction in Blount Disease.

Background: Blount disease is a disorder of the posteromedial proximal tibial physis which causes a progressive varus, procurvatum, and internal rotation deformity of the tibia. Untreated, it can cause significant limb malalignment. The goal of this study is to evaluate the results of correction of Blount disease using types of external fixation.

Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy.

Orthopedic care is an important aspect of the overall management of patients with Duchenne muscular dystrophy (DMD). In addition to progressive muscle weakness and loss of function, patients may develop joint contractures, scoliosis, and osteoporosis, causing fractures; all of these necessitate intervention by a multidisciplinary team including an orthopedic surgeon as well as rehabilitation specialists such as physio- and occupational therapists. The causes of these musculoskeletal complications are multifactorial and are related to primary effects on the muscles from the disease itself, secondary effects from weak muscles, and the related side effects of treatments, such as glucocorticoid use that affect bone strength.

IRF2BPL Is Associated with Neurological Phenotypes.

Interferon regulatory factor 2 binding protein-like (IRF2BPL) encodes a member of the IRF2BP family of transcriptional regulators. Currently the biological function of this gene is obscure, and the gene has not been associated with a Mendelian disease. Here we describe seven individuals who carry damaging heterozygous variants in IRF2BPL and are affected with neurological symptoms.

Pediatric proximal femur fractures.

Pediatric proximal femur fractures are rare injuries resulting from high-energy trauma. The Delbet classification is used when describing these injuries, and associates fracture type to the development of avascular necrosis. Historically, casting was utilized in the treatment of these injuries, but high complication rates following this approach have changed the treatment modality to early and anatomic fixation.

Analysis of the impact of race on blood transfusion in pediatric scoliosis surgery.

Introduction: Surgical correction of pediatric scoliosis is associated with significant blood loss. Minimizing estimated blood loss and blood transfusion is beneficial as transfusions have been associated with increased morbidity, including risk of surgical site infections, longer hospitalizations, and increased cost. Although there is evidence that African-American or Black adults are more likely to require intraoperative blood transfusion compared with Caucasian or White adults, the reasons for this difference are unclear.

Looking beyond the exome: a phenotype-first approach to molecular diagnostic resolution in rare and undiagnosed diseases.

PurposeTo describe examples of missed pathogenic variants on whole-exome sequencing (WES) and the importance of deep phenotyping for further diagnostic testing.MethodsGuided by phenotypic information, three children with negative WES underwent targeted single-gene testing.ResultsIndividual 1 had a clinical diagnosis consistent with infantile systemic hyalinosis, although WES and a next-generation sequencing (NGS)-based ANTXR2 test were negative.

An Investigation into the Use of Manufactured Sand as a 100% Replacement for Fine Aggregate in Concrete.

Manufactured sand differs from natural sea and river dredged sand in its physical and mineralogical properties. These can be both beneficial and detrimental to the fresh and hardened properties of concrete. This paper presents the results of a laboratory study in which manufactured sand produced in an industry sized crushing plant was characterised with respect to its physical and mineralogical properties.

Neuromuscular Scoliosis: Current Concepts.

Scoliosis is a common deformity in neuromuscular disorders. This spinal deformity usually presents at an early age, rapidly progresses during growth, and continues to progress even after skeletal maturity. Neuromuscular scoliosis classically involves the entire thoracolumbar spine, often extending to the pelvis and causing pelvic obliquity.

Osteonecrosis of the femoral head in childhood malignancy.

Purpose: Children undergoing chemotherapeutic treatment of malignancies have up to a 9 % incidence of osteonecrosis. The purpose of this article is to determine the time from initiation of chemotherapy to the onset of symptoms and the diagnosis of osteonecrosis of the femoral head in this patient population.

Methods: A retrospective review of the records of 18 patients (29 hips) under 21 years of age with both a diagnosis of osteonecrosis of the femoral head and childhood onset malignancy was undertaken to determine the time from initiation of chemotherapy to the onset of symptoms and diagnosis of osteonecrosis of the femoral head.

Radiographic outcomes of ring external fixation for malunion and nonunion.

Ring external fixators such as the Ilizarov external fixator (IEF) and Taylor Spatial Frame (TSF) have revolutionized management of limb deformity. This study examines radiographic outcomes for treatment of malunions and nonunions with secondary emphasis on comparing radiographic union rates of TSF and IEF. A retrospective chart and radiograph review was performed.

Distant Harrington rod migration 35 years after implantation.

Harrington rods have been successfully implanted in thousands of patients for the correction of scoliotic deformity since the 1950s. An exceedingly rare complication of Harrington rod placement is loosening with resultant migration. The authors present a 50-year-old woman who had a single Harrington rod placed when she was 15 years old.